Continuous R-DA-EDOCH alternated with high-dose Ara-C induces deep remission and overcomes high-risk factors in young patients with newly diagnosed mantle cell lymphoma.

Objective: Our previous studies have indicated potentially higher proliferative activity of tumor cells in Chinese patients with mantle-cell lymphoma (MCL) than those in Western. Given the success and tolerability of R-DA-EDOCH immunochemotherapy in treating aggressive B-cell lymphomas, we designed a prospective, phase 3 trial to explore the efficacy and safety of alternating R-DA-EDOCH/R-DHAP induction therapy for young patients with newly diagnosed MCL. The primary endpoint was the complete remission rate (CRR) at the end of induction (EOI).

Raman spectroscopy and bioinformatics-based identification of key genes and pathways capable of distinguishing between diffuse large B cell lymphoma and chronic lymphocytic leukemia.

Diffuse large B-cell lymphoma (DLBCL) and chronic lymphocytic leukemia (CLL) are subtypes of non-Hogkin lymphoma (NHL) that are generally distinct form one cases, but the transformation of one of these diseases into the other is possible. Some patients with CLL, for instance, have the potential to develop Richter transformation such that they are diagnosed with a rare, invasive DLBCL subtype. In this study, bioinformatics analyses of these two NHL subtypes were conducted, identifying key patterns of gene expression and then experimentally validating the results.

Mitochondrial respiratory complex IV deficiency recapitulates amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is categorized into ~10% familial and ~90% sporadic cases. While familial ALS is caused by mutations in many genes of diverse functions, the underlying pathogenic mechanisms of ALS, especially in sporadic ALS (sALS), are largely unknown. Notably, about half of the cases with sALS showed defects in mitochondrial respiratory complex IV (CIV).

Thalidomide-based regimen shows promising efficacy in large granular lymphocytic leukemia: a multicenter phase II study.

Large granular lymphocytic leukemia (LGLL) is characterized by the clonal proliferation of cytotoxic T lymphocytes or NK cells. Standard first-line immunosuppressive treatments have limitations, achieving complete remission (CR) rates of up to 50%. Immune system dysregulation is implicated in LGLL.

Classification of NK-large granular lymphocytic leukemia by CD56 expression.

NK-large granular lymphocytic leukemia (NK-LGLL) is a rare chronic lymphoproliferative disorder and displays heterogeneity that remains insufficiently defined. CD56 plays a pivotal role in NK-cell maturation linked to cytotoxicity. However, whether CD56 might be associated with distinctive characteristics in NK-LGLL has not been determined.

[Advances in complement inhibition therapy for paroxysmal nocturnal hemoglobinuria].

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematologic disorder. Historically, most PNH patients mainly received supportive treatment, which led to poor quality of life and high mortality rates. The advent of downstream complement C5 inhibitors has dramatically changed the natural course of PNH.

[Familial erythrocytosis type 2 due to VHL germline mutations: a case report and literature review].

To enhance the understanding of familial erythrocytosis type 2 (ECYT2) resulting from compound heterozygous mutations in the VHL gene. We conducted a retrospective analysis of the case data from a patient with ECYT2 to investigate its pathogenesis, clinical features, diagnosis and treatment options, as well as prognosis, while also reviewing the relevant literature. A 31-year-old man was admitted to the hospital due to facial and hand flushing that had persisted for 29 years.

[Analysis of risk factors for early death in hyperleukocytic acute leukemia].

This study analyzed the clinical characteristics and early mortality risk factors in patients with hyperleukocytic acute leukemia (HAL) to provide a basis for predicting early prognosis. Data were retrospectively collected from 211 patients with primary HAL who visited the Emergency Center of the Hematology Hospital, Chinese Academy of Medical Sciences, between July 1, 2019 and November 30, 2021. The value of each indicator in early risk stratification and prognosis was analyzed.

[The value of sequential organ failure assessment and its dynamic changes in predicting mortality in hematology intensive care unit].

To investigate the value of Sequential Organ Failure (SOFA) score and its dynamics (ΔSOFA) in predicting mortality in hematology care unit (HCU) . A retrospective clinical study was conducted on 79 critically ill hematologic patients admitted to the Center for Critical Care Medicine, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences, between May and June 2024. SOFA scores and ΔSOFA were calculated within 2 days before and after HCU admission.

[The state of the art: diagnosis and therapeutic interventions of clonal hematopoiesis of indeterminate potential and clonal cytopenias of undetermined significance].

Clonal hematopoiesis of indeterminate potential (CHIP) and clonal cytopenias of undetermined significance (CCUS) are included in both fifth edition of the World Health Organization classification (WHO 2022) and International Consensus Classification (ICC). Recently three models were developed for prediction of myeloid neoplasms risk and clinical trials are initiated to investigate potential treatments for CHIP and CCUS. Challenges in diagnosis and therapeutic intervention of CHIP and CCUS were discussed.

Enhancing the Stability of Sweet Protein Neoculin for Food Applications Using Computational Strategies.

Sweet proteins, known for their high sweetness and low caloric content, offer great potential as safe, low-calorie sweeteners without the negative health effects associated with sugar or artificial alternatives. Neoculin, unique for its dual properties of sweetness and taste-modifying, holds significant promise for food industry applications. However, its low thermal stability limits its broader use in food processing, highlighting the need to improve its stability.

Management of children and adolescents with chronic myeloid leukemia in chronic phase: International pediatric chronic myeloid leukemia expert panel recommendations.

The treatment strategy for children and adolescents with chronic myeloid leukemia in the chronic phase (CML-CP) has evolved from allogeneic hematopoietic stem cell transplantation (HSCT) to tyrosine kinase inhibitors (TKIs). With the advent of next-generation TKIs and new targeted therapies in the CML field, an international pediatric CML expert panel provides recommendations based on the medical literature (including previous pediatric guidelines), national standards, and treatment principles used in adults with CML-CP. Recommendations include diagnosis of the disease and details on managing the initial steps of care of children and adolescents with newly diagnosed CML-CP, including complications such as leukostasis.

Effect of Interpretation of Positive Metagenomic Next-Generation Sequencing Reports on the Infection Diagnosis in Patients With Hematological Disorders.

Background: Metagenomic next-generation sequencing (mNGS) has become a crucial diagnostic tool for infectious diseases in patients with hematological disorders. However, despite the abundant microbial information provided by positive mNGS reports, interpreting these results remains challenging due to the lack of standardized criteria.

Methods: We surveyed 92 clinicians to identify common challenges in understanding mNGS reports.

Chinese expert consensus on flow cytometric detection of hematological malignant cells in tissue samples.

Flow cytometry (FCM), characterized by its simplicity, rapid processing, multiparameter analysis, and high sensitivity, is widely used in the diagnosis, treatment, and prognosis of hematological malignancies. FCM testing of tissue samples not only aids in diagnosing and classifying hematological cancers, but also enables the detection of solid tumors. Its ability to detect numerous marker parameters from small samples is particularly useful when dealing with limited cell quantities, such as in fine-needle biopsy samples.

Abdominal obesity and frailty progression in population across different Cardiovascular-Kidney-Metabolic syndrome stages: a nationwide longitudinal study.

Background: Abdominal obesity, assessed via the body roundness index (BRI), is a critical determinant of health outcomes. This study explores the association between abdominal obesity and frailty progression across different stages of Cardiovascular-Kidney-Metabolic (CKM) syndrome in a nationwide longitudinal cohort.

Methods: Data were derived from the China Health and Retirement Longitudinal Study, including individuals aged ≥ 45 years.

Disrupted small-world architecture and altered default mode network topology of brain functional network in college students with subclinical depression.

Background: Subclinical depression (ScD), serving as a significant precursor to depression, is a prevalent condition in college students and imposes a substantial health service burden. However, the brain network topology of ScD remains poorly understood, impeding our comprehension of the neuropathology underlying ScD.

Methods: Functional networks of individuals with ScD (n = 26) and healthy controls (HCs) (n = 33) were constructed based on functional magnetic resonance imaging data.

[Hypoprothrombinemia-lupus anticoagulant syndrome: a case report and literature review].

The Hypoprothrombinemia-Lupus Anticoagulant Syndrome (HLAS) is a rare coagulation disorder, typically presenting with bleeding manifestations. It is characterized by decreased prothrombin activity and the presence of lupus anticoagulant, but laboratory findings are complex, which can delay diagnosis and treatment. This report describes the diagnosis and management of a HLAS patient who was an 11-year-old girl with recurrent bleeding.

[Acute promyelocytic leukemia with disseminated intravascular coagulation: a case report and literature review].

Acute promyelocytic leukemia (APL) is characterized by an aggressive onset and rapid progression. During induction chemotherapy, it is prone to being complicated by disseminated intravascular coagulation (DIC), which is a leading cause of early death in patients. This study reports a case of APL complicated by DIC during induction therapy with all-trans retinoic acid (ATRA) combined with arsenic trioxide (ATO).

[Acute myeloid leukemia with translocation t (8;16) (p11;p13) and prominent coagulation abnormalities at onset: case report and review of literature].

Acute Myeloid Leukemia with t (8;16) (p11;p13) is a rare subtype of AML. This article presents a retrospective analysis of a 19-year-old female patient with t (8;16) (p11;p13) AML, focusing on her clinical features and treatment course, alongside a review of relevant literature. The patient was admitted due to skin ecchymosis and gastrointestinal bleeding, rapidly progressing to disseminated intravascular coagulation.

[Acquired FX deficiency: a case report and literature review].

To introduce the clinical features, laboratory findings, diagnostic approaches, and treatment of acquired factor X deficiency. This study reviewed a case of primary light-chain amyloidosis with acquired factor X deficiency admitted to our hospital. The patient's clinical course and diagnostic process were described, and a literature review was conducted.

[Hereditary protein C deficiency presenting with predominant hemorrhagic symptoms: a case report and literature review].

To enhance the understanding of hereditary protein C deficiency. A case of a child with severe hereditary protein C deficiency, presenting with recurrent muscle bleeding as the primary clinical manifestation, was reported. The diagnostic and treatment were detailed, and relevant literature was reviewed.

Phylogeny-Informed Discovery of New Glycopeptide Antibiotics Produced by a Minimal Biosynthetic Gene Cluster.

Glycopeptide antibiotics (GPAs) are the frontline medicines to treat multidrug-resistant Gram-positive infections. Resistance to GPAs warrants the development of new antibiotics. Phylogeny reclassification of GPA biosynthetic gene clusters with a focus on the type V subgroup bearing a heptapeptide scaffold identified new GPAs, varsomycins, possessing unprecedented 3,5-dihydroxyphenylalanine at position 5 and an F-G biphenyl cross-link.